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Blepharospasm
| Image = Gray379.png | Caption = Left orbicularis oculi, seen from behind. | DiseasesDB = 15748 | ICD10 = | ICD9 = | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = oph | eMedicineTopic = 202 | MeshID = D001764 | }} A blepharospasm (from Greek: blepharo, eyelid, and spasm, (or bletharospasm or Bruegel's syndrome Beaumant J.G., Kenealy, P.M. & Rogers, M.J.C. (1999). The Blackwell Dictionary of Neuropsychology. Oxford:Blackwell) is an uncontrolled muscle contraction of the orbicularis muscles of the eyes, resulting in an abnormal tic or twitch of the eyelid which may seem similar to be voluntary winking. However unlike hemifacial spasm it is bilateral with no associated clonic twitching of the lower facial muscles.In severe cases the eye may be closed completely. It normally refers to Benign Essential Blepharospasm, a focal dystonia - a neurological movement disorder involving involuntary and sustained muscle contractions of the muscles around the eyes. Benign means the condition is not life threatening. Essential indicates that the cause is unknown, but fatigue, stress, or an irritant are possible contributing factors. Symptoms sometimes last for a few days then disappear without treatment, but in most cases the twitching is chronic and persistent, causing lifelong challenges. The symptoms are often severe enough to result in functional blindness. The person's eyelids feel like they are clamping shut and will not open without great effort. Patients have normal eyes, but for periods of time are effectively blind due to their inability to open their eyelids. Although strides have recently been made in early diagnosis, blepharospasm is often initially mis-diagnosed as allergies or "dry eye syndrome". It is a fairly rare disease, affecting only one in every 20,000 people in the United States. Symptoms *Excessive blinking and spasming of the eyes, usually characterized by uncontrollable eyelid closure of durations longer than the typical blink reflex, sometimes lasting minutes or even hours. *Uncontrollable tics or twitches of the eye muscles and surrounding facial area. Some sufferers have twitching symptoms that radiate into the nose, face and sometimes, the neck area. *Dryness of the eyes *Sensitivity to the sun and bright light Epidemiology The disorder is more comen in older women. There is also some evidence for a family history of the disorder.Beaumant J.G., Kenealy, P.M. & Rogers, M.J.C. (1999). The Blackwell Dictionary of Neuropsychology. Oxford:Blackwell Causes What causes Blepharospasm is largely unknown, although some educated guesses are being made. In most cases, blepharospasm seems to develop spontaneously. Some blepharospasm patients have a previous history of dry eyes and/or light sensitivity, but others report no previous eye problems before onset of initial symptoms. It may be associated with disorders of the pyramidal tractBeaumant J.G., Kenealy, P.M. & Rogers, M.J.C. (1999). The Blackwell Dictionary of Neuropsychology. Oxford:Blackwell Blepharospasm may also come from abnormal functioning of the brain's basal ganglia. Concomitance with dry eye, as well as other dystonias such as Meige's syndrome has been observed. Blepharospasms can be caused by concussions in some rare cases, when a blow to the back of the head damages the basal ganglia. Some drugs can induce blepharospasm, such as those used to treat Parkinson's disease, as well as sensitivity to hormone treatments, including Estrogen replacement therapy for women going through Menopause; blepharospasm can also be a sympton of acute withdrawal from benzodiazepine dependence. Treatment *Drug therapy: Drug therapy for blepharospasm has proved generally unpredictable and short-termed. Finding an effective regimen for any patient usually requires trial and error over time. In some cases a dietary supplement of magnesium chloride has been found effective. *Botulin toxin (Botox) injections have been used, to induce localized, partial paralysis. Among most sufferers, botox is the preferred treatment method. Injections are generally administered every three months, with variations based on patient response and usually give almost immediate relief (though for some it may take more than a week), from the spasming. Most patients can resume a relatively normal life with regular Botox treatments. A minority of sufferers get minimal or no result from Botox injections and have to find other treatments. For some, Botox diminishes in its effectiveness after many years of use. An observed side effect in a minority of patients is ptosis or eyelid droop. Attempts to inject in locations that minimise ptosis can result in diminished ability to control spasms. *Surgery: Patients that do not respond well to medication or botulinum toxin injection are candidates for surgical therapy. The most effective surgical treatment has been protractor myectomy, the removal of muscles responsible for eyelid closure. *Dark glasses are often worn because of sunlight sensitivity, as well as to hide the eyes from others. *Stress management and support groups can help sufferers deal with the disease and prevent social isolation. References *Alkin, T., Onur, E., & Ozerdem, A. (2007). Co-occurence of blepharospasm, tourettism and obsessive-compulsive symptoms during lamotrigine treatment: Progress in Neuro-Psychopharmacology & Biological Psychiatry Vol 31(6) Aug 2007, 1339-1340. *Alonso-Navarro, H., & Jimenez-Jimenez, F. J. (2006). Tardive Blepharospasm Associated With Cinnarizine Use: Clinical Neuropharmacology Vol 29(4) Jul-Aug 2006, 187-189. *Assael, M. (1967). Hysterical Blepharospasm: Diseases of the Nervous System 28(4) 1967, 256-258. *Bhatia, M. S., & Gupta, R. (2006). Letters: Essential Blepharospasm Responding to Haloperidol: Journal of Neuropsychiatry & Clinical Neurosciences Vol 18(4) Fal 2006, 554-556. *Bihari, K., Pigott, T. A., Hill, J. L., & Murphy, D. L. (1992). Blepharospasm and obsessive-compulsive disorder: Journal of Nervous and Mental Disease Vol 180(2) Feb 1992, 130-132. *Brantley, P. J., Carnrike, C. L., Faulstich, M. E., & Barkemeyer, C. A. (1985). Blepharospasm: A case study comparison of trihexyphenidyl (Artane) versus EMG biofeedback: Biofeedback & Self Regulation Vol 10(2) Jun 1985, 173-180. *Brasic, J. R., Zagzag, D., Kowalik, S., Prichep, L., Barnett, J. Y., Bronson, B., et al. *Broocks, A., Thiel, A., Angerstein, D., & Dressler, D. (1998). Higher prevalence of obsessive-compulsive symptoms in patients with blepharospasm than in patients with hemifacial spasm: American Journal of Psychiatry Vol 155(4) Apr 1998, 555-557. *Defazio, G., Martino, D., Abbruzzese, G., Girlanda, P., Tinazzi, M., Fabbrini, G., et al. (2007). Influence of coffee drinking and cigarette smoking on the risk of primary late onset blepharospasm: Evidence from a multicentre case control study: Journal of Neurology, Neurosurgery & Psychiatry Vol 78(8) Aug 2007, 877-879. *Dening, T. R. (1987). Blinking and essential blepharospasm: Comprehensive Psychiatry Vol 28(1) Jan-Feb 1987, 83-92. *Diamond, E. L., Trobe, J. D., & Belar, C. D. (1984). Psychological aspects of essential blepharospasm: Journal of Nervous and Mental Disease Vol 172(12) Dec 1984, 749-756. *Duggal, H. S., & Mendhekar, D. N. (2007). 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A behavioural treatment of blepharospasm: Two case reports: Behavioural Psychotherapy Vol 10(2) Apr 1982, 184-188. *Gulati, S., Singh, A. N., & Libretto, S. E. (2003). Successful risperidone rechallenge after blepharospasm in a patient with schizophrenia: 24-month follow-up: Journal of Psychopharmacology Vol 17(4) Dec 2003, 453-454. *Johnstone, S. J., & Adler, C. H. (1998). Headache and facial pain responsive to Botulinum toxin: An unusual presentation of blepharospasm: Headache: The Journal of Head and Face Pain Vol 38(5) May 1998, 366-368. *Kagohashi, M., Okuma, Y., Fujishima, K., Kitada, T., & Mizuno, Y. (2004). Blepharospasm associated with multiple system atrophy: A case report and review of the literature: Parkinsonism & Related Disorders Vol 10(3) Mar 2004, 169-171. *Lee, Y., Yeh, W.-C., Chong, M.-Y., Lin, P.-Y., & Chang, Y.-Y. (2007). Venlafaxine and tardive blepharospasm: A case report: Progress in Neuro-Psychopharmacology & Biological Psychiatry Vol 31(5) Jun 2007, 1139-1140. *Leon-Sarmiento, F. E., Bayona-Prieto, J., & Gomez, J. (2005). Letter to the Editor: Neurophysiology of blepharospasm and multiple system atrophy: Clues to its pathophysiology: Parkinsonism & Related Disorders Vol 11(3) May 2005, 199-201. *Levin, H., & Reddy, R. (2000). Clozapine in the treatment of neuroleptic-induced blepharospasm: A report of 4 cases: Journal of Clinical Psychiatry Vol 61(2) Feb 2000, 140-143. *Maurri, S., & Barontini, F. (1990). Do psychopathologic aspects exist in essential blepharospasm? : Rivista di Psichiatria Vol 25(2) Apr-Jun 1990, 93-97. *Micheli, F., Cersosimo, G., Scorticati, M. C., Ledesma, D., & Molinos, J. (1999). Blepharospasm and apraxia of eyelid opening in lithium intoxication: Clinical Neuropharmacology Vol 22(3) May-Jun 1999, 176-179. *Mostofsky, D. I., Yehuda, S., Rabinovitz, S., & Carasso, R. (2000). 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Botulinum toxin improves the quality of life and reduces the intensification of depressive symptoms in patients with blepharospasm: Parkinsonism & Related Disorders Vol 13(8) Dec 2007, 505-508. *Okuma, Y., Kagohashi, M., & Mizuno, Y. (2005). Reply to letter to the editor: Neurophysiology of blepharospasm in multiple system atrophy: Parkinsonism & Related Disorders Vol 11(3) May 2005, 203-204. *Onuf, B. (1907). Spasm of the Apparatus of Binocular Fixation and Superinduced Blepharospasm in a Hysterical Patient with a Theory of their Pathogenesis: The Journal of Abnormal Psychology Vol 2(4) Oct-Nov 1907, 155-165. *Peck, D. F. (1977). The use of EMG feedback in the treatment of a severe case of blepharospasm: Biofeedback & Self Regulation Vol 2(3) Sep 1977, 273-277. *Pfersmann, D., Pockberger, H., & Zapotoczky, H. G. (1987). Behaviour therapy in the treatment of blepharospasm: Nervenarzt Vol 58(6) Jun 1987, 383-387. *Poewe, W., Benke, T., Felber, S., & Aichner, F. (1989). 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External links *Blepharospasm Resource Guide from the National Eye Institute (NEI). *BEBRF: Benign Essential Blepharospasm Research Foundation This organization is a very important link - especially for those who may first be realizing they have blepharospasm. They provide comprehensive and up-to-date information about the disease, links to many informative articles on the subject, as well as support group contacts in most parts of the country. But most importantly, they provide a bulletin board for sufferers to discuss both challenges and helpful information about the disease. http://www.blepharospasm.org/forums/beb/index.html *Blepharospasm, Steen-Hall Eye Institute Category:Neurology